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患有对扎努布鲁丁尼布有反应的disialosyl抗体的慢性无氧神经病变
Benedetta Tierro1, Andrea Visentin2, Alessandro Salvalaggio1
1Neurology Unit, Department of Neurosciences, University of Padova, Padova, Italy.
European journal of neurology
|February 24, 2026
概括
本案例研究详细介绍了一名患有瓦尔登斯特罗姆相关慢性无氧神经病变与迪沙洛西尔抗体 (CANDA) 的患者,他在采布鲁丁尼布治疗后经历了显著的临床和神经生理恢复.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 血液学 血液学 血液学
背景情况:
- 具有迪沙洛西尔抗体 (CANDA) 的慢性动脉毒性神经病是罕见的神经疾病.
- 沃尔登斯特罗姆的巨型球蛋白血症可能与CANDA有关.
- 治疗耐药性在CANDA中很常见,治疗选择有限.
研究的目的:
- 报告一个与Waldenström相关的CANDA病例.
- 描述临床过程和治疗反应.
- 为了突出扎努布鲁丁尼布在治疗耐药的CANDA患者中的疗效.
主要方法:
- 一名62岁的男性患者患有感官症状,并被诊断为与Waldenström的大球蛋白血症相关的CANDA.
- 最初的治疗包括静脉注射免疫球蛋白 (IVIg) 和修复药,反应不佳.
- 随后,该患者接受了 Bruton's tyrosine kinase (BTK) 抑制剂尚布鲁丁尼布的治疗,并与IVIg结合治疗.
主要成果:
- 患者显示了总体临床改善和结束剂量波动的解决与zanubrutinib治疗.
- 15个月后,观察到显著的血液学和神经生理学改善.
- 抗disialosyl抗体 (GD1a,GD1b) 持续存在,而抗化体抗体 (GM1,GM2) 变得无法检测.
结论:
- 这是第一例报告的Waldenström相关的CANDA病例,证明了zanubrutinib的益处.
- 赞努布鲁丁尼布为对其他治疗不耐药的CANDA患者提供了潜在的治疗选择.
- 这些发现表明,BTK抑制可能有效地管理CANDA,即使具有持久抗体.
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